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Cufence (trientine dihydrochloride) is a small molecule copper-chelating agent developed by Univar BV for the treatment of Wilson disease. Wilson disease is an inherited autosomal recessive disorder caused by mutations in the ATP7B gene, leading to the toxic accumulation of copper in the liver and brain. Cufence is specifically indicated for the treatment of Wilson disease in patients five years and older who are intolerant to D-penicillamine therapy. The drug works by binding to free systemic copper to form a stable complex that is subsequently excreted through the urine. Additionally, trientine may inhibit the intestinal absorption of dietary copper. It is available as 300 mg capsules and was granted marketing authorization in the European Union.
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